捷克首次对1887例意义未明的单克隆丙种球蛋白病病例进行分析。

A first Czech analysis of 1887 cases with monoclonal gammopathy of undetermined significance.

作者信息

Sandecká Viera, Hájek Roman, Pour Luděk, Špička Ivan, Ščudla Vlastimil, Gregora Evžen, Radocha Jakub, Walterová Lenka, Kessler Petr, Zahradová Lenka, Adamová Dagmar, Valentova Kamila, Vonke Ivan, Obernauerová Jarmila, Starostka David, Wróbel Marek, Brožová Lucie, Jarkovský Jiří, Mikulášová Aneta, Říhová Lucie, Ševčíková Sabina, Straub Ján, Minařík Jiří, Adam Zdeněk, Krejčí Marta, Král Zdeněk, Maisnar Vladimír

机构信息

Department of Internal Medicine, Hematology and Oncology, University Hospital, Brno, Czech Republic.

Department of Clinical Hematology, University Hospital, Ostrava, Czech Republic.

出版信息

Eur J Haematol. 2017 Jul;99(1):80-90. doi: 10.1111/ejh.12894. Epub 2017 May 11.

DOI:10.1111/ejh.12894

PMID:28384387

Abstract

INTRODUCTION

Monoclonal gammopathy of undetermined significance (MGUS) is a premalignant condition with a risk of malignant conversion.

PATIENTS AND METHODS

With the aim to estimate the cumulative risk MGUS progression to hematologic malignancies, we analyzed a nationwide population-based cohort of 1887 MGUS patients from the Czech Registry of Monoclonal Gammopathies (RMG) between 2007 and 2013.

RESULTS

During the follow-up period (median 4 years; range 0.6-34.8), progression to hematologic malignancies was observed in 8.6% (162 of 1887) of patients. Factors associated with progression were as follows: M-protein concentration ≥1.5 g/dL, pathological sFLC (<0.26 or >1.65) ratio, bone marrow plasma cells (BMPCs) in cytology >5%, immunoparesis, age ≥69 years, and the level of serum hemoglobin at baseline <12.0 g/dL. Combining these factors, we propose a new risk model (CMG model). The risk of progression at 10 years was 1.6%, 16.9%, 22.9%, 39.4%, and 52.3%, respectively, if 0 (reference group), one, two, three, or four to five risk factors are present (P<.001) with HR 63 times higher compared to the reference MGUS group.

CONCLUSION

The new CMG model was established with an advantage for better identification of MGUS patients at low risk.

摘要

引言

意义未明的单克隆丙种球蛋白病（MGUS）是一种具有恶性转化风险的癌前病变。

患者与方法

为了评估MGUS进展为血液系统恶性肿瘤的累积风险，我们分析了2007年至2013年间来自捷克单克隆丙种球蛋白病登记处（RMG）的1887例MGUS患者的全国性人群队列。

结果

在随访期间（中位时间4年；范围0.6 - 34.8年），8.6%（1887例中的162例）的患者进展为血液系统恶性肿瘤。与进展相关的因素如下：M蛋白浓度≥1.5 g/dL、病理性游离轻链（sFLC）（<0.26或>1.65）比值、骨髓细胞学中的浆细胞（BMPCs）>5%、免疫球蛋白缺乏、年龄≥69岁以及基线时血清血红蛋白水平<12.0 g/dL。综合这些因素，我们提出了一种新的风险模型（CMG模型）。如果存在0个（参照组）、1个、2个、3个或4至5个风险因素，10年时进展的风险分别为1.6%、16.9%、22.9%、39.4%和52.3%（P<0.001），与参照MGUS组相比，风险比（HR）高63倍。